Miscellaneous disorders affecting skin and joints

Several syndromes have been described which incorporate hyperelasticity of skin with joint hypermobility. The commonest is Marfan’s syndrome. Affected individuals are tall, with spindly digits (arachnodactyly). The skin may have an abnormal doughy texture and joint hypermobility is common. Cutaneous manifestations are more prominent in various forms of Ehlers—Danlos syndrome (EDS), the main forms of which are listed in Table 9.1. Cutaneous features include hyperextensible skin, skin fragility and bruising, ‘cigarette paper’ scarring, striae and molluscoid pseudotumours (Fig. 9.1). In type IV EDS distended blood vessels are apparent on the chest wall, associated with acrogeria and a high incidence of rupture of great vessels and bowel. Joint hypermobility is marked and is the principal feature of EDS type III. Hypermobility is complicated by recurrent dislocations and subsequent osteoarthritis. Thin skin and joint laxity are common features of osteogenesis imperfecta.